CASE REPORT
Solving Acne Inversa (Hidradenitis Suppurativa) in Crohn Disease with Buried Chip Skin Grafts Oliver Bleiziffer, Adrian Dragu, Ulrich Kneser, and Raymund E. Horch Background: Acne inversa is a progressive chronic inflammatory disease associated with abscess and fistula formation and subsequent scarring. In recent years, an increasing number of reports have been published about acne inversa and concomitantly occurring Crohn disease. Extensive anogenitoperineal soft tissue defects represent an enormous challenge to therapy. Common treatment strategies of reconstructive surgery such as local flaps, free flaps, or split-thickness skin grafts are frequently problematic and associated with poor outcomes. Objective: The aim of this case report is to demonstrate the clinical problem of extensive anogenitoperineal soft tissue defects and to present a surgical technique that can be successfully used in such difficult cases. Methods: In both cases, extensive excision of the affected areas was carried out down to the gluteal muscles followed by intramuscular grafting of buried skin chip grafts. Results: In both cases, the final postoperative results after numerous surgeries were very good, with complete wound closure resulting in complete recovery and social reintegration. Conclusion: The technique of buried skin chip grafting has long been ignored since split-thickness skin grafting and different techniques of flap coverage evolved, but it may constitute a highly efficient treatment strategy in challenging reconstructive situations. Ante´ce´dents: L’acne´ inversa est une maladie inflammatoire chronique et progressive, associe´e a` des abce`s et a` des formations fistuleuses donnant lieu a` des cicatrices. Au cours des dernie`res anne´es, de plus en plus de rapports sont parus au sujet de l’acne´ inversa et de l’apparition simultane´e de la maladie de Crohn. D’importants dommages au tissu mou de la re´gion anoge´nitope´rine´ale posent d’e´normes de´fis pour le traitement. Les strate´gies de traitement au moyen de la chirurgie reconstructive les plus souvent suivies, telles que les lambeaux ou la greffe de peau fine, produisent de mauvais re´sultats. Objectif: De´montrer, graˆce a` deux cas, les proble`mes cliniques lie´s aux dommages conside´rables cause´s au tissu mou de la re´gion anoge´nitope´rine´ale et pre´senter une technique chirurgicale qui peut eˆtre utilise´e avec succe`s dans les cas difficiles. Me´thodes: Chez les deux patients, une importante excision des re´gions affecte´es a e´te´ effectue´e, s’e´tendant aux muscles glute´aux, suivie d’une greffe enfouie intramusculaire. Re´sultats: Dans les deux cas, les re´sultats finaux apre`s plusieurs ope´rations e´taient tre`s bons, avec cicatrisation comple`te qui a mene´ a` une re´mission totale et a` la re´inte´gration sociale. Conclusion: La technique de greffe enfouie a e´te´ longtemps ignore´e, surtout avec l’e´volution de la greffe de peau douce et autres techniques. Toutefois, la greffe enfouie peut repre´senter une strate´gie de traitement hautement efficace dans les situations de reconstruction complique´es.
From the Department of Plastic and Hand Surgery, University of Erlangen-Nu¨rnberg, Medical Center, Erlangen, Germany. Presented in part as a poster at the annual meeting of the German Society of Plastic Surgeons in Aachen, Germany, October 2006, and published in the meeting’s abstract supplement. Address reprint requests to: Oliver Bleiziffer, MD, Department of Plastic and Hand Surgery, University of Erlangen-Nu¨rnberg, Medical Center, Krankenhausstrasse 12, 91054 Erlangen, Germany; e-mail: oliver.
[email protected].
DOI 10.2310/7750.2008.08008 # 2009 Canadian Dermatology Association
164
HE CLINICAL COURSE of Crohn’s disease can be aggravated by numerous extraintestinal manifestations in almost every organ system, including the skin.1 Typical cutaneous manifestations of Crohn disease include formation of fissures and fistulae involving skin and mucosa.2 In recent years, an increasing number of reports directed attention to a different skin condition that could be misdiagnosed as a cutaneous manifestation of Crohn disease, acne inversa (also known as hidradenitis suppurativa), which has recently been reported to occur in
T
Journal of Cutaneous Medicine and Surgery, Vol 13, No 3 (May/June), 2009: pp 164–168
Solving Acne Inversa in Crohn Disease
coincidence with Crohn disease at a higher incidence than one would randomly expect.3,4 Acne inversa is a chronic and progressive inflammatory disease of the apocrine glands associated with recurrent formation of deep abscesses. Young adults are affected in the majority of cases. The prevalence of the disease is between 1:100 and 1:600, with women being affected two to five times more often than men. Although the axillary region is most commonly affected in females, anogenital manifestation is particularly frequent in male patients.3,4 The long-term course of the condition is often aggravated by and associated with scarred deep sinus tract fistulae, fluctuant draining of abscesses, and excruciating pain.3,4 Although early acute cases in which the involved area is limited can be treated with incision and drainage of the abscess, more severe cases with deep scarring and formation of fistulae require surgical excision of the affected area.5 In cases of very extensive acne inversa in the anogenital area, however, where primary wound closure cannot be accomplished, permanent defect coverage can become a significant clinical challenge. In this report, two patients with a long-standing history of concomitant Crohn disease and acne inversa are presented: a 39-year old man with a 13-year history and a 57-year-old man with a 20-year course of disease. Transplantation of buried chip skin grafts, in which skin samples are harvested from the wound margins and minced into chip grafts measuring 1 to 2 cm followed by insertion into the freshly excised wound bed with a thin forceps, is discussed as a valuable treatment option for this challenging combination of clinical problems.
Case Reports Case 1 A 39-year-old otherwise healthy man presented with a 13year history of Crohn disease and coincidental extensive acne inversa. The patient had undergone multiple incisions of abscesses and operations in the past and had been on long-term immunosuppressive therapy. He complained of severe pain and psychological distress owing to the unsightly aspect of multiple abscesses and a foul smell and presented to our plastic and reconstructive surgery clinic for treatment options. Clinical examination revealed an extensive area of nonhealing wounds, including the perianal, gluteal, sacral, and scrotal areas, the proximal part of the thighs, and the inguinal region (Figure 1A).
165
After typical fistula formation owing to Crohn disease was excluded, radical excision of a 55 3 60 cm area was performed initially. At the same time, skin transplantation was carried out using skin chip grafts, which were buried intramuscularly. Wound margins were approximated down to the excised muscles, and the preserved anal opening was sutured down to the surrounding musculature as well (Figure 1B). Skin samples were harvested from the wound margins and minced into chip grafts measuring 1 to 2 cm, which were then buried into the freshly excised wound bed with a thin forceps. The wound was left open to allow for healing per secondary intention supported by the skin chip grafts. The patient was discharged 16 days later when his condition had already significantly improved as granulation tissue formation had proceeded rapidly and infection of the wound was no longer detectable. At this time, signs of small sprouting skin islands from the buried chips were already visible (Figure 1, C and D). The wound was de´brided again 3 weeks later, again followed by intramuscular transplantation of multiple buried chip skin grafts, which were freshly harvested from the wound margin and processed similar to the initial surgery. During follow-up visits, further progression of granulation tissue formation was observed in the wound. There were recurrences of abscess-forming infections in the inguinal region and in the perineal and scrotal areas, which were treated by de´bridement and intramuscular implantation of buried chip skin grafts. Infection was completely eradicated, all wounds healed by secondary intention, and the patient has not suffered any relapse for 2K years despite ongoing immunosuppression with steroids and, occasionally, methotrexate (Figure 1E). There was no formation of unstable scars, where, for any reason, there is frequent loss of skin coverage over the scar, which constitutes the main concern when the buried chip skin graft technique is applied. The patient declined our offer to further reduce the scars, which had already decreased in size, by multiple transposition flap procedures. Case 2 A 56-year-old man with an over 30-year history of Crohn disease and acne inversa presented with extensive involvement of the gluteal, lumbar, and perianal areas and the inner thigh region. Similar to patient 1, he had been on long-term immunosuppressive therapy and complained of severe pain and psychological distress (Figure 2A). Clinical examination revealed extensive nonhealing wounds in the gluteal, lumbal, perianal, and inner thigh regions with multiple fistulae where drainage of pus was visible.
166
Bleiziffer et al
Figure 1. Patient 1. A, Preoperative view at initial presentation. An extensive area of nonhealing wounds can be appreciated, as well as signs of abscess formation of different stages, including past abscesses where incisions had resulted in scarring. B, Initial postoperative views after radical excision of a 55 3 60 cm area as a first step of surgical treatment. At the same time, autologous skin samples were harvested and minced into chip grafts measuring 1 to 2 cm, which were then buried into the freshly excised wound bed with a thin forceps. C, The freshly buried chip skin grafts can be identified as the white spots in the wound ground. D, Two weeks after initial de´bridement and buried chip skin grafting, the wound condition has significantly improved, and rapid progression of granulation tissue formation can be seen, as well as sprouting of skin islands arising from the buried chip skin grafts transplanted at the time of the initial de´bridement. E, Final result after a total of three surgeries and repetitive transplantation of buried chip skin grafts. The wound was completely healed, without formation of unstable scars—the main concern after transplantation of buried chip skin grafts.
In a procedure analogous to that described for case 1, wide radical complete excision of the affected areas was performed together with implantation of buried skin chip grafts, as described in detail above (Figure 2B). Five weeks postoperatively, small epithelial islands could be detected arising from the transplanted chip skin grafts (Figure 2, C and D). At this time, the decision was made to repeat the procedure. Hence, repeated transplantation of buried chip skin grafts was performed after modest de´bridement. Three and a half months after the second procedure, the wounds were almost completely epithelialized, and fistula formation or unstable scars could not be detected (Figure 2E). No relapse has occurred since then, despite ongoing immunosuppression.
Discussion Skin involvement is a relatively common problem in Crohn disease and occurs in around 9 to 20% of all cases.6 Our patients developed massive skin changes in the
perianal, gluteal, and anogenital areas. When perianal and genital abscesses, fistulae, swelling, and scar formation occur in a patient suffering from Crohn disease, it is tempting to classify these findings as complications of the underlying clinical problem. The present case reports demonstrate that acne inversa, which appears to occur at a higher incidence in patients with Crohn disease, can cause similar changes and must be discriminated from Crohn disease in terms of differential diagnosis. Acne inversa is characterized by formation of persisting and recurring nodules, painful abscesses, deep scarring, and extended fistulae, which are frequent sources of bacterial infection and subsequent pus production.3 The condition is believed to be partially genetic and inherited on an autosomal dominant basis.7 Its prevalence was reported to be 4.1%.8 The first report about three cases of concomitant acne inversa in patients with Crohn disease was published 15 years ago.9 In the following years, a number of authors published reports about single cases or small series of
Solving Acne Inversa in Crohn Disease
167
Figure 2. Patient 2. A, Preoperative view. A large area of abscess- and fistula-forming wounds involving the posterior thigh, lower back, and anogenital and perineal areas. B, Postoperative view 1 week after radical excision of all involved areas. Minced chip skin grafts were harvested and processed followed by intramuscular transplantation as described above. C, Five weeks after surgery. D, The buried chip skin grafts have proliferated into small skin islands, which can be identified as white spots in the wound ground in a detailed view. E, Result 7 months after transplantation of buried chip skin grafts. Epithelial islands have grown to a subtotally confluent layer of skin, resulting in an almost completely healed wound.
patients in whom diagnosis of both diseases could be established.6,10 The diagnosis is based on the history and clinical examination and is difficult to verify, particularly in the early stages of the disease. At a later time point, the clinical picture of complex fistulae in combination with the bridging scars becomes so characteristic that the diagnosis can usually be established based on these findings. Although the typical locations where acne inversa is encountered include the axillary, inguinal, and perianal areas, Crohn disease–associated acne is primarily—and in many cases exclusively—found in the anogenital region, as in both patients presented in this report. Superinfections with bacteria are present on a regular basis in these situations. Surgical excision of the affected skin and its appendages is the only treatment that can offer long-term therapeutic success in acne inversa.3,5,11 Abscess incision is frequently
performed but fails to provide a lasting cure. Attempts at conservative therapy with isotretinoin can provide temporary relief only in early-stage cases or are used for preconditioning prior to surgical therapy.12,13 Owing to the fact that the disease is still relatively little known, it is common for patients like the ones we present in this report to be misdiagnosed and inefficiently treated for years before curative surgical intervention is finally performed.12 The 3-year recurrence rate was reported to be 27% for radical wide excision compared with 45% for local excision and 100% when only an incision was carried out.5 This study by Ritz and colleagues emphasizes the need for wide surgical excision of the compromised area to provide lasting relief and prevent recurrences. The coverage of soft tissue defects in the perineal, gluteal, and perianal regions poses a challenge to the reconstructive surgeon for the following reasons: high local
168
Bleiziffer et al
bacterial contamination, mechanical forces owing to functional requirements (ie, in the sitting position), impossibility of immobilization, paucity of local flaps, and significant pain. In this case report, we present two patients with 55 3 60 cm soft tissue defects that, owing to their size and location, were characterized by high bacterial contamination, significant pain, strain induced by mechanical forces, and the absence of options for local or distant flap coverage. An ongoing immunosuppressive regimen owing to Crohn disease constituted an additional unfavorable influence on wound healing. Although alternative treatment strategies such as flap coverage or split-thickness skin grafting are often unsuccessful and result in loss of the transplanted skin, intramuscular implantation of buried chip grafts resulted in complete restoration of skin integrity in both cases, along with social reintegration of the patients. We previously reported successful application of the buried chip skin graft technique for treatment of perianal burn wounds14 and, in combination with vacuum-assisted closure therapy, for treatment of diabetic ulcers in the lower extremity.15 Graft survival is not disturbed by mechanical irritation when stool is wiped off the wound since the grafts are buried within the muscle. Replacement of skin and other tissues has been an area of intense investigation in recent years,16–20 but tissueengineered skin substitutes have their disadvantages owing to cumbersome in vitro cultivation and expansion and high costs.17 The buried chip skin graft technique is inexpensive, easy to use, and efficient. In our opinion, the time to achieve wound closure is more than justified given the very good final result and the fact that repetitive, time-consuming, and technically demanding operations can be avoided.
3. 4. 5.
6. 7.
8.
9. 10.
11. 12. 13. 14.
15.
16. 17. 18.
References 1. Lembcke B, Kruis W, Sartor R. Systemic manifestations of IBD: the pending challenge for subtle diagnosis and treatment. Dordrecht: Kluwer Academic; 1998. 2. Friedman S, Blumberg R. Inflammatory bowel disease. In: Fauci AS, Braunwold E, Kasper DL, et al, editors. Harrison’s principles
19. 20.
of internal medicine. 7th ed. New York: McGraw-Hill; 2008. p. 1886–99. Plewig G, Kligman A. Acne inverse. In: Plewig G, Kligman A, editors. Acne and rosacea. 3rd ed Berlin: Springer; 2000. p. 309–41. Brown TJ, Rosen T, Orengo IF. Hidradenitis suppurativa. South Med J 1998;91:1107–14. Ritz JE, Runkel L, Haier J, et al. Extent of surgery and recurrence rate of hidradenitits suppurativa. In J Colorectal Dis 1998;13:164– 8. Goischke HK, Ochsendorf FR. Acne inverse in Crohn’s disease. Z Gastroenterol 2001;39:965–9. von der Werth JM, Williams HC, Raeburn JA. The clinical genetics of hidradenitis suppurativa revisited. Br J Dermatol 2000;142:947– 53. Jemec GB, Heidenheim M, Nielsen NH. The prevalence of hidradenitis suppurativa and its potential precursor lesions. J Am Acad Dermatol 1996;35:191–4. Ostlere LS, Langtry JA, Mortimer P, et al. Hidradenitis suppurativa in Crohn’s disease. Br J Dermatol 1991;125:384–6. Church J, Fazio V, Lavery I, Oakley J, et al. The differential diagnosis and comorbidity of hidradenitis supurativa and perianal Crohn’s disease. Int J Colorectal Dis 1993;8:117–9. Seelig MH, Moser TH, Schonleben K. Pyodermia fistulans sinifica. Chir Praxis 1998;54:23–31. Roedder-Wehrmann O, Kuester W, Plewig G. Acne inversa. Hautarzt 1991;42:5–8. Jansen T, Plewig G. Acne inversa. Fortschr Med 1998;116:46–8. Horch RE, Stark GB, Spilker G. Behandlung der perianalen Verbrennungen mit versenkten Hautpartikeln. Zentralbl Chir 1994;119:722–5. Kopp J, Kneser U, Bach AD, et al. Buried chip skin grafting in neuropathic diabetic foot ulcers following vacuum-assisted wound bed preparation: enhancing a classic surgical tool with novel technologies. In J Low Extrem Wounds 2004;3:168–71. Horch RE. Future perspectives in tissue engineering. J Cell Mol Med 2006;10:4–6. Bleiziffer O, Eriksson E, Yao F, et al. Gene transfer strategies in tissue engineering. J Cell Mol Med 2007;11:206–23. Bach AD, Arkudas A, Tjiawi J, et al. A new approach to tissue engineering of vascularized skeletal muscle. J Cell Mol Med 2006; 10:716–26. Fiegel HC, Kaufmann PM, Bruns H, et al. Review: hepatic tissue engineering. J Cell Mol Med 2007;[Epub ahead of print]. Kneser U, Schaefer DJ, Polykandriotis E, et al. Tissue engineering of bone: the reconstructive surgeon’s point of view. J Cell Mol Med 2006;10:7–19.
